Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis

The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distin...

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Bibliographic Details
Published in:Life (Basel, Switzerland) Switzerland), 2022-09, Vol.12 (10), p.1467
Main Authors: Konstantouli, Afroditi Maria, Lioulios, Georgios, Stai, Stamatia, Moysidou, Eleni, Fylaktou, Asimina, Papagianni, Aikaterini, Stangou, Maria
Format: Article
Language:English
Subjects:
ANCA-associated vasculitis
Antibodies
Antigens
Antineutrophil cytoplasmic antibodies
Classification
clinical phenotype
Clinical trials
Cytokines
Epidemiology
Gender
Granulomatosis
Immune system
Leukocytes (eosinophilic)
myeloperoxidase
Neutrophils
Nomenclature
outcome
Pathogenesis
Phenotypes
proteinase 3
Review
Ultraviolet radiation
Vasculitis
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Summary:The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distinct clinical entities. This classification has proved its expedience in clinical trials and everyday clinical practice; yet, a substantial overlap in clinical presentation still exists and often causes difficulties in prompt definition and clinical distinction. Additionally, new insights into the AAV pathogenesis point out that PR3 and MPO-AAV may not represent expressions of the same disease spectrum but rather two distinct disorders, as they display significant differences. Thus, it is supported that a classification based on ANCA serotype (PR3-ANCA, MPO-ANCA or ANCA-negative) could be more accurate and also closer to the nature of the disease compared to the phenotype-based one. This review aims to elucidate the major differences between PR3 and MPO-AAV in terms of epidemiology, pathogenesis, histological and clinical manifestations and response to therapeutic approaches.
ISSN:2075-1729
2075-1729
DOI:10.3390/life12101467